Right Atrial Extension of Primary Venous Leiomyosarcoma: Pulmonary Embolism and Budd-Chiari Syndrome at Presentation

Abstract

Venous leiomyosarcomas are rare and arise predominantly in the inferior vena cava (IVC). The clinical findings are nonspecific and may precede the diagnosis by several years. IVC leiomyosarcoma is predominantly seen in women. Intracavitary extension of vascular tumors tends to result from embolization or propagation along great veins, and this is a serious risk factor for pulmonary embolism and sudden death when it reaches the right heart. Modern imaging modalities using computed tomography, magnetic resonance imaging, individually or in combination with cavography, ultrasound, and echocardiography, allow an early and accurate preoperative diagnosis, resulting in a higher rate of surgical resection and improved survival. The authors present a 72-year-old woman who presented with pulmonary embolism and Budd-Chiari syndrome. Pathological examination revealed a leiomyosarcoma. The tumor, involving the IVC, was diagnosed with imaging techniques that showed intracardiac extension of a primary venous leiomyosarcoma. The literature discussing leiomyosarcoma of the IVC is briefly reviewed.